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XERODERMA PIGMENTOSA

 

This is a very rare disorder in which patients are exquisitely sensitive to light.

 

This results in premature ageing of skin and skin cancers.

In the first stage at infancy multiple pigmented (blackish) spots appear over the exposed area of skin.  In the next stage the skin becomes wrinkled and appears wrinkly and appears old.  Later in the last stage skin cancers appear.  Other features include decreased cortical function, hearing problems.  The defect is in the repair process after sun exposure.  Normally each cell is able to repair sun induced damage.  In xeroderma the repair mechanisms are faulty.

 The treatment remains

  1. Sun protection
  2. Strict sun protection
  3. Even more strict sun protection

 This helps to delay all the effects.

 A dedicated family and understanding patient can survive all the vagaries of this problem

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